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Early development of an infant with 18q- syndrome.

The early intellectual and psychological development of an infant with 18q- syndrome is described. In this syndrome, which is a rare chromosomal disorder, there is a deletion of a portion of the long arm of chromosome 18. The few previously published accounts have indicated a varying degree of intellectual retardation. This child proved to be moderately delayed only and her development over the first 24 months of age is described in some detail. The family was receiving an intensive home advisory service offered to help maximize the development of pre-school handicapped infants in the area, and it is suggested that in general terms this particular disorder is not remarkably different in respect of psychological prognosis from the more common Down's syndrome, involving trisomy 21, and shows an equally favourable response to such a service. The peculiar appearance of such infants may, however, pose particular problems of parental adjustment.

Abnormalities, Multiple↗

Not by bread alone project: a 2-year follow-up report.

BACKGROUND: We have previously reported the developmental gains achieved, after introducing a simple programme of structured play to stimulate children in an orphanage. It was envisaged that the caregivers could continue the programme. However, the enthusiasm of the caregivers waned over the year the programme was entrusted to them. After 1 year, a full time play therapist was recruited to rejuvenate the play programme. METHODS: Children's development was assessed using the Indian adaptation of the Bayley Scales of Infant Development. The first assessment was done when the play therapist joined. Subsequently, three-monthly assessments were done and the scores achieved were recorded. RESULTS: The initial mean motor and mental scores, when the play therapist joined, were 66.14 and 56.95, respectively (similar to the pre-intervention scores of the pilot study reported in an earlier paper). The scores improved to 81.84 and 78.25 within 3 months of restarting the play programme. CONCLUSION: The schedule of the 'Not by Bread Alone' project can accelerate the motor and mental development of children in orphanages. However, it requires a highly motivated and dedicated person to sustain this programme over long periods.

Attitude to Health↗

To look or not to look? Typical and atypical development of oculomotor control.

The ability to inhibit saccades toward suddenly appearing peripheral stimuli (prosaccades) and direct them to contralateral locations instead (antisaccades) is a crucial marker of eye movement control. Typically developing infants as young as 4-month-olds can learn to inhibit reflexive saccades to peripheral stimuli, but they do not produce antisaccades, whose development later in infancy and its underlying neural computations remain unexplored. Here we tested oculomotor control in typically developing toddlers and toddlers with fragile X syndrome (FXS), a disorder of known genetic origin that allows the investigation of the neuro-computational properties contributing to the development of saccadic control. Typically developing toddlers decreased looking toward peripheral cues that predicted contralateral rewards, whose appearance they anticipated. Furthermore, this correlated with age, indicating a gradual development of saccadic control. In contrast with the typical case, toddlers with FXS did not decrease their looks to peripheral onsets that predicted contralateral events. Importantly, the atypical pattern of performance was also evident in the elimination of the correlation with mental or chronological age found in controls. Taken together, the findings suggest that control of saccades and its developmental trajectory is atypical in toddlers with FXS, consistent with inhibitory deficits previously shown at later ages in this condition. Potential implications for the neural mechanisms underlying the typical and atypical development of oculomotor control are discussed.

Age Factors↗

Neurological, electrophysiological and MRI abnormalities in infants with extensive cystic leukomalacia.

Twenty infants, diagnosed by cranial ultrasound as having extensive cystic leukomalacia, had visual evoked responses (VER) and electroencephalograms (EEG) in the neonatal period and MRI scans later in infancy. The early ultrasound findings and results from the electrophysiological tests were correlated with later MRI findings and functional abilities. In infants with periventricular leukomalacia (PVL), the cysts were usually no longer visible by ultrasonography, beyond 40 weeks postmenstrual age (PMA), but later MRI scans showed a consistent pattern of delayed myelination around the irregularly dilated occipital horns of the lateral ventricles. VER's were present in the neonatal period and vision was maintained, although all infants developed a marked squint. EEG's were either normal or abnormal initially, but improvement was noted within several weeks. In those with subcortical or mixed lesions, cysts were noted to persist beyond 40 weeks PMA. Later MRI scans showed very poor myelination, with poor progress on subsequent scans and cortical atrophy. VER's were absent and all infants later became cortically blind. EEG's were severely abnormal and recovery was very poor. The infants with PVL developed spastic diplegia with moderate developmental delay, while those with mixed or subcortical lesions developed quadriplegia with severe mental retardation. An integrated approach, consisting of ultrasound imaging and electrophysiological recordings in the neonatal period and MRI imaging later in infancy, may provide a more reliable prediction of the pattern of later deficits.

Brain↗

The incidence of renal calcification in preterm infants.

A total of 79 infants born at less than 32 weeks' gestation were studied with serial renal ultrasound scans to assess the incidence of nephrocalcinosis. Twenty one infants developed renal calcification giving an overall incidence of 26.6% in the study group. Affected infants were significantly smaller (mean (SD) birth weight 940 (323) g) and significantly less mature (mean (SD) gestation 26.9 (1.9) weeks). In 17 patients the calcification was represented by hyperechogenic renal pyramids alone, and in four patients renal calculi were demonstrated. Factors associated with renal calcification included hypophosphataemia, hypercalcaemia, hypercreatininaemia, and prolonged oxygen requirement during the first month of life. Multivariate analysis showed that the strongest clinical indicator of calcification was duration of oxygen treatment. Infants who still required oxygen treatment at 28 days had a 62% chance of developing renal calcification.

England↗

Amiloride-sensitive nasal potential difference is not changed by estradiol and progesterone replacement but relates to BPD or death in a randomized trial on preterm infants.

Postnatal replacement of placental estradiol (E2) and progesterone (P) in preterm infants may improve lung function, possibly mediated through enhanced epithelial Na(+) transport and alveolar fluid clearance. Preterm infants of <29 wk gestational age and <1000 g birth weight requiring mechanical ventilation within 12 h of birth were randomized to receive either 2.5 mg/kg E2 and 22.5 mg/kg P per day (E2/P), or vehicle placebo. Epithelial Na(+) transport was assessed in 29 infants by measuring total nasal potential difference (NPD) and amiloride-sensitive NPD (ASNPD) on postnatal days of life 1, 3, 5, and 7, and mean values of all four measurements were calculated. Bronchopulmonary dysplasia (BPD) was defined as need for supplemental oxygen (goal Sa(O2) 90%) or mechanical ventilation at 36 wk corrected postmenstrual age. Mean ASNPD was -6.5 +/- 2.8 mV in infants receiving E2/P and -6.1 +/- 2.6 mV in infants receiving placebo (not significant). NPD was -10.6 +/- 3.8 mV and -10.7 +/- 3.6 mV, respectively. The ASNPD was significantly higher in infants surviving without BPD (-7.1 +/- 2.5 mV) than in infants developing BPD or not surviving (-5.2 +/- 2.4 mV). In conclusion, ASNPD is not changed by postnatal replacement of E2 and P. Infants at high risk of developing BPD had lower ASNPD values in the immediate postnatal period.

Amiloride↗

Information processing and developmental assessments in 3-month-old infants exposed prenatally to cocaine.

OBJECTIVE: To determine the effect of prenatal cocaine exposure on 3-month infant information processing and developmental assessments. METHODS: One hundred and eight infants, 61 cocaine-exposed and 47 controls, participated at 3 months of age in an infant-control habituation and novelty responsiveness procedure and in a developmental assessment using the Bayley Scales of Infant Development both administered by experimenters blind to the drug exposure status of the infant. RESULTS: Compared to the non-drug-exposed group, infants exposed prenatally to cocaine were significantly more likely to fail to start the habituation procedure and, for those who did, significantly more likely to react with irritability early in the procedure. The majority of infants in both groups reached the habituation criterion, and among those who did there were no significant differences between cocaine and non-cocaine-exposed infants in habituation or in recovery to a novel stimulus. Infants who were cocaine-exposed showed comparatively depressed performance on the motor (Psychomotor Developmental Index) but not the mental (Mental Developmental Index (MDI)) scales of the Bayley. These results obtained for habituation and Bayley MDI controlling for both perinatal and sociodemographic factors. CONCLUSIONS: Differences in reactivity to novelty but not in information processing between cocaine-exposed and non-cocaine-exposed infants suggest that the effects of prenatal cocaine exposure may be on arousal and attention regulation rather than early cognitive processes.

Adult↗

Predictors of neurodevelopmental outcome of Malaysian very low birthweight children at 4 years of age.

OBJECTIVE: To determine neonatal, early developmental and social risk factors that predict the neurocognitive and behavioural outcome of very low birthweight (VLBW) preschool children at four years of age. METHODOLOGY: From a cohort of 151 eligible VLBW survivors born in Kuala Lumpur Maternity Hospital, 116 (76.8%) were prospectively followed up from birth till four years. A standardised neurological examination was performed at one and four years to determine the presence of impairment and cerebral palsy, respectively. Cognitive development was assessed using the Mental Scale of the Bayley Scales of Infant Development (MDI) at one year and the Weschler Preschool and Primary Scale of Intelligence-Revised (WIPPSI-R) at four years. Motor coordination was assessed using the Movement Assessment Battery for Children (Movement-ABC). Mothers completed the Child Behaviour Checklist (CBCL) and Parenting Stress Index (PSI) questionnaires. Logistic and multiple regression analyses were used to determine factors associated with cerebral palsy, IQ scores, Movement-ABC and CBCL scores. RESULTS: Factors associated with cerebral palsy were lower MDI scores at one year (P = 0.001) and late neonatal cranial ultrasound abnormalities (P = 0.036). Minor (P = 0.016) or major impairment (P = 0.003) at one year of age and a low level of paternal education (P = 0.01) were associated with poor motor function on the Movement-ABC scale. Lower levels of maternal education (P < 0.001), impairment at one year (P = 0.002) and late neonatal cranial ultrasound abnormalities (P = 0.039) predicted Full Scale IQ scores. Higher PSI scores (P = 0.001), younger mothers (P = 0.003) and late neonatal cranial ultrasound abnormalities (P = 0.009) were associated with worsened child behaviour scores on the CBCL scale. CONCLUSION: Social factors and the caregiving environment were important determinants of cognitive and behavioural outcome. Cranial ultrasound abnormalities in the late neonatal period and the developmental status at one year might be useful in identifying high risk infants in need of long-term surveillance.

Child Development↗

Parent-child interactions and development of toddlers born preterm.

Fathers and mothers of 49 healthy preterm and 54 full-term infants were observed interacting with their child at 3 and 12 months using the Nursing Child Assessment Teaching Scale. Each parent completed the Parenting Stress Index at both times. At 18 months adjusted age, the children were assessed using the Bayley Scales of Infant Development, Sequenced Inventory of Communication Development-Revised, and MacArthur Communicative Development Inventory. Preterm children scored significantly lower on the Bayley Mental scale and on the number of words produced. Early parent-child interactions contributed to the child's development. Mother's interactions with the child, child gender, and family socioeconomic status predicted 17% of the variance in the Bayley Mental score. Mother's and father's interactions with the child, child gender, and the child's behavior with his or her mother predicted 22% of the variance in receptive communication skills.

Adult↗

trans Fatty acids: infant and fetal development.

This review evaluates scientific data associated with the possibility that trans fatty acids compromise fetal and infant early development. Concerns have been triggered by research that has heightened our awareness of the importance of n-3 and n-6 fatty acids; shown that trans fatty acids inhibit delta6 desaturation of linoleic acid; identified trans fatty acid isomers in fetal, infant, and maternal tissues; and reported an inverse association between the trans fatty acid content of tissue lipids and measures of growth and development. Animal studies provide little evidence that trans fatty acids influence growth, reproduction, or gross aspects of fetal development. However, these models may not have been appropriate for addressing all the subtle effects that influence development of human infant retinal, neural, or brain function. Human studies are hampered by the complexity of the interrelations among nutritional, genetic, and environmental factors and by ethical considerations that constrain the research design. Existing data have not established a causal relation between trans fatty acid intake and early development. Conclusions cannot be drawn from the possible association found between trans fatty acid exposure and lower n-3 and n-6 long-chain polyunsaturated fatty acids and growth because of confounding factors. Few studies addressed the question of whether trans fatty acids adversely affect human fetal growth. One study reported a correlation between the trans fatty acid content of plasma and birth weight of preterm infants and one study reported a relation between preterm births and the trans fatty acid content of maternal plasma. Limited associative data have addressed whether trans fatty acids adversely affect fetal and infant neurodevelopment and growth. The interpretation of existing research and development of recommendations should be done cautiously. Suggestions for research to clarify these issues are made.

Child↗

Dental enamel in relation to ionized calcium and parathyroid hormone. Studies of human primary teeth and rat maxillary incisors.

The aims of this thesis were to evaluate the role of lowered calcium values in blood on enamel formation and mineralization, and further to analyze whether the calcium regulating parathyroid hormone (PTH) would have any effects on the forming and mineralizing enamel. DENTAL ENAMEL AND IONIZED CALCIUM: Human primary teeth from two groups of infants were clinically and histologically examined. One group (n = 25) was born with optimal perinatal conditions. The infants had low, but not hypocalcemic values on day 1 when compared with days 3 and 5 postpartum. The second group of infants (n = 11) was subjected to blood exchange transfusion on the first few days postpartum. All infants developed very low values of blood ionized calcium due to the treatment, and the infants had a mean of three hypocalcemic days. Enamel aberrations were seen in both groups, but only four infants had multiple enamel aberrations located at levels corresponding to the enamel development at the time of the birth. Low values of blood ionized calcium alone were not associated with enamel aberrations, as the aberrations were only seen in infants subjected to more than three blood exchange transfusions. The neonatal line was present in all teeth investigated, thin lines dominated, and the widths were not dependent on the levels of blood ionized calcium during the first few days after birth. Rats were also used to investigate whether the maxillary incisor enamel would be affected during a diet-induced hypocalcemic state. Of ten experimental rats, nine had normal enamel, indicating that hypocalcemia in rats does not generally affect the enamel. DENTAL ENAMEL AND PARATHYROID HORMONE: Rats was used in a study where they were subjected to daily injections of PTH. Three different doses were used and the experimental periods were 7 and 14 days. In a pilot part, enamel aberrations were seen but not in the main study performed later. The main difference in experimental design between the pilot and the main part of the study was the use of hard tissue marker, oxytetracycline, at start of the pilot part. For this reason, a separate study was performed to evaluate the use of oxytetracycline in hard tissue research. The results clearly demonstrated that oxytetracycline itself induces enamel aberrations and so it was not used in the main study of parathyroid hormone. As no enamel aberrations were seen in the main part, it was concluded that the doses of PTH used do not seem to affect enamel formation and mineralization.

Amelogenesis↗

The effect of thyroxine treatment started in the neonatal period on development and growth of two-year-old Down syndrome children: a randomized clinical trial.

CONTEXT: Young Down syndrome children appear to have a mild form of congenital hypothyroidism that is rarely detected by neonatal screening and usually left untreated. OBJECTIVE: To investigate the effects of thyroxine treatment on development and growth of young Down syndrome children. DESIGN, SETTING, AND PARTICIPANTS: Single-center, randomized, double-blind, 24-month trial (enrollment June 1999 to August 2001) with nationwide recruitment, comparing thyroxine administration with placebo in 196 Down syndrome neonates. INTERVENTION: Neonates were randomly assigned to treatment for 2 yr with either thyroxine (n = 99; initial dose 8 microg/kg) or placebo (n = 97). Daily thyroxine doses were adjusted at regular intervals to maintain plasma TSH in its normal and free T(4) concentrations in its high-normal range. Placebo dose adjustments mirrored those of thyroxine. MAIN OUTCOME MEASURES: The primary outcome was mental and motor development at age 24 months, assessed with the Bayley Scales of Infant Development II. RESULTS: At age 24 months, the developmental testing results of 90 thyroxine-, and 91 placebo-treated children were available for analysis. The thyroxine-treated children had a 0.7-month smaller delay in motor developmental age (95% confidence interval, -1.4 to 0), corresponding to a difference of seven motor developmental index points. The mental developmental age delay was also 0.7 month smaller in the thyroxine group (95% confidence interval, -1.5 to 0.2), but lacked statistical significance. Thyroxine-treated children had greater gains in length (1.1 cm; 95% confidence interval, 0.2 to 2.0) and weight (378 g; 95% confidence interval 55 to 701). CONCLUSIONS: The data of our study provide evidence to support the hypothesis that thyroxine treatment may improve development and growth of young Down syndrome children. Thyroxine treatment should be considered in Down syndrome neonates to maximize their early development and growth.

Apgar Score↗

Neurodevelopmental outcome of premature infants after antenatal phenobarbital exposure.

OBJECTIVE: We previously demonstrated that antenatal phenobarbital does not decrease the risk of intracranial hemorrhage or early death in premature infants. The objective of the present study was to evaluate the impact of antenatal phenobarbital exposure on the neurodevelopmental outcome of premature infants born to women who were participating in the randomized clinical trial of antenatal phenobarbital exposure. STUDY DESIGN: Infants were evaluated at 18 to 22 months corrected age with a standard neurologic examination and the Bayley scales of infant development measuring the mental developmental index and the psychomotor developmental index. RESULTS: Of the 578 infants <34 weeks of gestational age who were born to women who were enrolled in the primary study, 7 infants died after discharge from the neonatal intensive care unit, and 135 infants were lost to follow-up. Infants who were lost to follow-up had a higher mean birth weight and gestational age and a lower maternal education, but the rates of intracranial hemorrhage were comparable to those infants who were evaluated. Among the infants who were evaluated (n = 436; 76%), the mean birth weight and gestational age, maternal education, and frequency and distribution of intracranial hemorrhage were similar in the antenatal phenobarbital exposed and placebo groups. Eighteen infants (8%) in the antenatal phenobarbital exposed group and 21 infants (11%) in the placebo group had cerebral palsy (P = not significant). There was no difference between the 2 groups in either the median Bayley II mental developmental index (85 in the antenatal phenobarbital and 86 in the placebo group) or the Psychomotor Developmental Index (91 in the antenatal phenobarbital and 91 in the placebo group). Infants with intracranial hemorrhage (23%) had significantly lower mental developmental index and psychomotor developmental index scores than infants with no intracranial hemorrhage, independent of antenatal phenobarbital exposure. In the total cohort of 436 infants, the presence of intracranial hemorrhage or periventricular leukomalacia was associated with lower mental developmental index and psychomotor developmental index scores; the presence of increasing birth weight, maternal education, and a complete course of antenatal steroids was associated with a higher mental developmental index score. CONCLUSION: Antenatal phenobarbital exposure did not favorably or adversely affect the neurodevelopmental outcome of premature infants at 18 to 22 months of age.

Anticonvulsants↗

Early versus late treatment of posthaemorrhagic ventricular dilatation: results of a retrospective study from five neonatal intensive care units in The Netherlands.

UNLABELLED: Posthaemorrhagic ventricular dilatation (PHVD) in very preterm infants carries a poor prognosis. As earlier studies have failed to show a benefit of early intervention, it is recommended that PHVD be first treated when head circumference is rapidly increasing and/or when symptoms of raised intracranial pressure develop. Infants with PHVD, admitted to 5 of the 10 Dutch neonatal intensive care units were studied retrospectively, to investigate whether there was a difference in the time of onset of treatment of PHVD and, if so, whether this was associated with a difference in the requirement of a ventriculo-peritoneal (VP) shunt and/or neurodevelopmental outcome. The surviving infants with a gestational age <34 wk, born between 1992 and 1996, diagnosed as having a grade III haemorrhage according to Papile on cranial ultrasound and who developed PHVD were included in the study. PHVD was defined as a ventricular index (VI) exceeding the 97th percentile according to Levene (1981), and severe PHVD as a VI crossing the p 97 + 4 mm line. Ninety-five infants met the entry criteria. Intervention was not deemed necessary in 22 infants, because of lack of progression. In 31 infants lumbar punctures (LP) were done before the p 97 + 4 mm line was crossed (early intervention). In 20/31 infants, stabilization occurred. In 9 a subcutaneous reservoir was placed, with subsequent stabilization in 6. In 5/31 infants a VP shunt was eventually inserted. In 42 infants treatment was started once the p 97 + 4 mm line was crossed (late intervention). In 30 infants LPs were performed and in 17 of these a VP shunt was eventually inserted. In 11 infants a subcutaneous reservoir was immediately inserted and in 8 of these infants a VP shunt was needed. In one infant a VP shunt was immediately inserted, without any other form of treatment. Infants with late intervention crossed the p 97 + 4 mm earlier (p 0.03) and needed a shunt (26/42; 62%) more often than those with early intervention (5/31; 16%). Early LP was associated with a strongly reduced risk of VP-shunting (odds ratio = 0.22, 95% confidence interval: 0.08-0.62). The number of infants who developed a moderate or severe handicap was also higher (11/42; 26%) in the late intervention group, compared with those not requiring any intervention (3/22; 14%) or treated early (5/31; 16%). CONCLUSION: In this retrospective study, infants receiving late intervention required shunt insertion significantly more often than those treated early. A randomized prospective intervention study, comparing early and late drainage, is required to further assess the role of earlier intervention.

Cerebral Hemorrhage↗

Infant vocalizations in response to speech: vocal imitation and developmental change.

Infants' development of speech begins with a language-universal pattern of production that eventually becomes language specific. One mechanism contributing to this change is vocal imitation. The present study was undertaken to examine developmental change in infants' vocalizations in response to adults' vowels at 12, 16, and 20 weeks of age and test for vocal imitation. Two methodological aspects of the experiment are noteworthy: (a) three different vowel stimuli (/a/, /i/, and /u/) were videotaped and presented to infants by machine so that the adult model could not artifactually influence infant utterances, and (b) infants' vocalizations were analyzed both physically, using computerized spectrographic techniques, and perceptually by trained phoneticians who transcribed the utterances. The spectrographic analyses revealed a developmental change in the production of vowels. Infants' vowel categories become more separated in vowel space from 12 to 20 weeks of age. Moreover, vocal imitation was documented, infants listening to a particular vowel produced vocalizations resembling that vowel. A hypothesis is advanced extending Kuhl's native language magnet (NLM) model to encompass infants' speech production. It is hypothesized that infants listening to ambient language store perceptually derived representations of the speech sounds they hear which in turn serve as targets for the production of speech utterances. NLM unifies previous findings on the effects of ambient language experience on infants' speech perception and the findings reported here that short-term laboratory experience with speech is sufficient to influence infants' speech production.

Age Factors↗

Comparison of cord blood immunoglobulin E concentrations and maternal allergy for the prediction of atopic diseases in infancy.

Total serum IgE levels were determined in 136 newborns and their mothers and in 54 of their fathers, using the paper radioimmunosorbent test (PRIST) technique. IgE specific antibodies for house dust (Dermatophagoides pteronyssinus), orchard grass, timothy grass, and cow's milk were measured with the radioallergosorbent test (RAST). One hundred thirty-three RAST assays were negative in newborns, and in three cases RAST for cow's milk was positive. Cord blood IgE ranged from 0 to 5.5 IU/ml (mean 0.32 +/- 0.54 IU/ml); levels were significantly (p less than 0.05) higher when maternal IgE was over 100 IU/ml and when mothers had received progesterone therapy during the pregnancy. Salbutamol administration or tobacco smoking during pregnancy did not influence newborn IgE. A clinical follow-up study was conducted in 83 infants for 9 mo. Nine infants developed definite atopic disease, and possible allergic diseases were noted in eight other infants. The IgE level at birth appeared to be more predictive for the development of allergy in infancy than the family history.

Albuterol↗

Assessment of motor development and function in preschool children.

The process of identification of children with delays or disorders in motor development includes developmental screening, examination, and reexamination. Throughout this process, various types of measures are used, including discriminative and evaluative measures. Discriminative and evaluative measures of motor development and function that are commonly used for preschool-aged children include the Bayley Scales of Infant Development II, Peabody Developmental Motor Scales, 2nd edition, Toddler and Infant Motor Evaluation, Pediatric Evaluation of Disability Inventory, and Gross Motor Function Measure. Selecting an appropriate measure is a crucial part of the examination process and should be geared toward the purpose of testing and characteristics of the child. Evidence of reliability and validity are important considerations for selection of a measure.

Child Development↗